Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly

Ilaria Del Giudice, Stefano A. Pileri, Maura Rossi, Elena Sabattini, Cristina Campidelli, Irene Della Starza, Maria S. De Propris, Francesca Mancini, Maria P. Perrone, Paola Gesuiti, Daniele Armiento, Luisa Quattrocchi, Agostino Tafuri, Angela Amendola, Francesca R. Mauro, Anna Guarini, Robin Foà

Research output: Contribution to journalArticle

Abstract

Five cases of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly are reported; three were splenectomized. BCL2/IGH rearrangements were found in three cases; HLA-DRB1*07 in all. Bone marrow (BM) trephines showed a moderate lymphoid infiltrate with intrasinusoidal distribution resembling a splenic marginal-zone lymphoma. Splenic white pulp revealed an enlargement of the marginal-zone area; red pulp was infiltrated by the same lymphocytes engulfing the sinuses. Splenic and BM B-lymphocytes were CD79a+/CD20+/IgM+/IgD+/bcl-2 +/CD27+/DBA.44-/CD31- and polyclonal by immunophenotype/polymerase chain reaction. PPBL features an expansion of splenic marginal-zone B-lymphocytes, which infiltrate BM sinusoids and circulate in the blood with no evidence of clonality, even in cases with progressive splenomegaly.

Original languageEnglish
Pages (from-to)726-731
Number of pages6
JournalBritish Journal of Haematology
Volume144
Issue number5
DOIs
Publication statusPublished - Mar 2009

    Fingerprint

Keywords

  • Histopathology
  • Lymphoproliferative disease
  • MZL
  • PPBL
  • Splenectomy

ASJC Scopus subject areas

  • Hematology

Cite this

Del Giudice, I., Pileri, S. A., Rossi, M., Sabattini, E., Campidelli, C., Starza, I. D., De Propris, M. S., Mancini, F., Perrone, M. P., Gesuiti, P., Armiento, D., Quattrocchi, L., Tafuri, A., Amendola, A., Mauro, F. R., Guarini, A., & Foà, R. (2009). Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly. British Journal of Haematology, 144(5), 726-731. https://doi.org/10.1111/j.1365-2141.2008.07551.x